Pulmonary Hypertension Treatment
in Hyderabad, India
PACE Hospitals is recognized as one of the best hospitals for pulmonary hypertension treatment in Hyderabad, Telangana, India. Our experienced team of pulmonologists and cardiologists work together to provide personalized care for patients diagnosed with pulmonary hypertension—a condition that affects the blood vessels in the lungs and puts strain on the heart.
We use a wide range of advanced diagnostic techniques such as echocardiography, right heart catheterization, CT pulmonary angiogram (CTPA), and blood tests to determine the exact cause and severity of the disease. Our goal is to ensure accurate diagnosis and timely intervention.
Our treatment strategies are tailored to each patient and may include pulmonary vasodilators, anticoagulants, diuretics, oxygen therapy, lifestyle modifications, and, in selected cases, surgical options like atrial septostomy or lung transplantation. We focus on timely diagnosis, slowing disease progression, improving heart-lung function, and enhancing patients' quality of life.
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Pulmonary Hypertension Treatment
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PACE Hospitals
HITEC City and Madeenaguda
Hyderabad, Telangana, India.
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Appointment Desk: 04048486868
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Regards,
PACE Hospitals
HITEC City and Madeenaguda
Hyderabad, Telangana, India.
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Pulmonary Hypertension Diagnosis
Diagnosis of Pulmonary hypertension is difficult because the symptoms of pulmonary hypertension (PH) are like those of other heart or lung conditions. Therefore, diagnosis may sometimes be delayed until a correct diagnosis is made. Pulmonary hypertension needs to be considered in individuals with chronic illness whose symptoms are out of proportion to the underlying disease or who do not respond well to standard treatments.
The pulmonologist considers the following factors before selecting the appropriate tests intended for the diagnosis of pulmonary hypertension:
- Clinical history
- Physical examination
Clinical history
- Dyspnoea on exertion, or shortness of breath during activity, is the most common and important symptom of pulmonary hypertension (PH).
- Additional common symptoms include syncope or near-syncope (fainting or feeling faint), which are serious symptoms that need immediate medical attention, and angina (chest pain), which develops when the heart muscle does not get enough oxygen.
- Fatigue, exercise intolerance, and a history of PH-related medical conditions are possible additional symptoms.
- Patients with unexplained or disproportionate symptoms need to have their clinical suspicion of PH raised, particularly if they do not improve after receiving standard treatments.
Physical examination
- Pulmonologists perform a physical examination to identify signs that may aid in diagnosing the condition. As part of this examination, the following are considered:
- Abnormal oxygen levels, which can be detected using pulse oximetry
- Elevated jugular venous pressure (Increased pressure in the right atrium due to poor venous return).
- Lower extremity edema (swelling in the legs and ankles, caused by fluid buildup due to right-sided circulatory congestion.)
- Right ventricular heaves (indicating increased right ventricular workload)
- Tricuspid regurgitation murmur (suggesting the backward flow of blood from the right ventricle into the right atrium dysfunction.)
- Signs of right heart failure, including hepatomegaly (enlarged liver) and ascites (abdominal fluid accumulation) due to gastrointestinal congestion.
Diagnostic evaluation of pulmonary hypertension
Pulmonary hypertension diagnosis requires a thorough evaluation by a pulmonologist. Several tests and procedures can help confirm the diagnosis and determine its severity. The following are the diagnostic tests for pulmonary hypertension:
- Initial noninvasive tests
- Blood tests (to rule out other causes)
- Electrocardiogram (ECG OR EKG)
- Chest X-ray
- Key screening test
- Transthoracic echocardiography
- Additional diagnostic tests
- Lung function tests
- Sleep study
- Exercise tests
- Advanced imaging
- Computed Tomography (CT) scan
- Cardiovascular Magnetic Resonance (CMR)
- Ventilation/perfusion (V/Q) scan
- Confirmatory test
- Right heart catheterization
- Specialized tests (if indicated)
- Genetic testing
- Lung biopsy
Initial Noninvasive Tests
Blood tests: Basic laboratory panels, including blood tests for renal function, liver function (LFTs), iron profiles, and thyroid studies, need to be performed in all patients with clinical signs of pulmonary hypertension (PH). Thyroid function tests should be performed on a regular basis, particularly if a patient's condition worsens, as thyroid dysfunction is frequently associated with pulmonary hypertension and can occur at any stage of the illness.
Routine HIV screening, hepatitis virus serologies, and connective tissue disease (CTD) tests need to be performed. Brain natriuretic peptide (BNP) and N-terminal pro-BNP (NT-pro-BNP) are included in the overall evaluation process, as they are valuable markers that can independently help predict outcomes.
Elevated antinuclear antibodies (ANAs) occur frequently, although in low titres (1:80). Recommended serological testing for scleroderma includes ANAs (as ELISA can be associated with false-negative tests, ANA immunofluorescence is recommended and should be considered positive at ≥1:160). If there is a high index of suspicion, an extended panel should be considered that includes anticentromere, anti-topoisomerase, anti-RNA polymerase III, double-stranded DNA, anti-Ro, anti-La, and U1-RNP antibodies.
Electrocardiograms (ECG or EKG): An electrocardiogram (ECG) in pulmonary hypertension helps identify electrical changes in the heart and can aid in detecting heart damage or excessive workload. In pulmonary hypertension, the heart may become strained because of damage or changes in the pulmonary arteries.
Chest x-ray: This is used to check an enlarged heart or scarring in the lungs, which may contribute to shortness of breath. It is also used to evaluate the size and shape of a heart and surrounding blood vessels, including the pulmonary arteries.
Key Screening Test
Transthoracic echocardiography: It is the most crucial noninvasive assessment tool for pulmonary hypertension. However, an echocardiographic assessment is recommended to estimate the probability of pulmonary hypertension. It uses the high frequency of sound waves to create an image of the heart, and it is used to calculate the pressure in the pulmonary arteries and tests how well both sides of the heart are pumping.
Additional Diagnostic Tests
Lung imaging tests: These tests play an important role in the diagnosis and treatment of pulmonary hypertension, which provides valuable insights into the causes, severity, and prognosis of the condition. The tests include:
- Pulmonary function tests: Pulmonary function tests, which include spirometry and the diffusing capacity for carbon monoxide, are recommended in patients with pulmonary hypertension to evaluate the underlying cause of the condition. Diffusing capacity is usually reduced in patients with pulmonary hypertension. These tests can show an obstructive pattern, which may suggest COPD, or a restrictive pattern indicating an interstitial lung disease. Pulmonary function tests also help to determine the severity of lung problems by giving both qualitative and quantitative data.
Overnight sleep study: By measuring sleep cycles and stages, it can detect sleep apnea, a condition where breathing frequently stops and starts during sleep. Identifying sleep apnea is crucial when evaluating pulmonary hypertension, as it can exacerbate or worsen the condition.
Exercise tests: A useful noninvasive method for identifying pulmonary hypertension is cardiopulmonary exercise testing (CPET), which identifies abnormal hemodynamics and ventilatory inefficiency related to both resting and exercise-induced pulmonary hypertension. Right heart catheterization is still necessary for a definitive diagnosis; however, CPET helps distinguish PH from other causes of exercise intolerance, evaluates the severity of the disease, and provides prognostic information.
Advanced Imaging
Computed Tomography (CT): It is also known as a CAT scan (computed axial tomography), provides clear images of the lungs and can help identify blood or other structural abnormalities. It is also used in evaluating PH to detect issues such as pulmonary embolism or blood clots in lung tissue or blood vessels.
Cardiovascular magnetic resonance (CMR): It is very sensitive and helps to evaluate heart wall movements and blood flow. It provides a comprehensive view of the heart and pulmonary vessels, enclosing both their structure and function. However, imaging the heart's structure requires high spatial resolution to visualize detailed anatomy and high temporal resolution to accurately capture motion and blood flow.
Ventilation/perfusion (V/Q) scan: This test measures the amount of air and blood flow in the lungs and is commonly used to check for blood clots that may cause pulmonary hypertension. This is crucial in the evaluation of pulmonary hypertension (PH), as untreated blood clots in the lungs may lead to elevated pressure in the pulmonary arteries, thereby worsening the condition and affecting heart and lung functions.
Confirmatory Test
Right cardiac catheterization
This is an invasive test used to confirm a diagnosis and obtain information that guides therapy decisions. The procedure involves inserting a catheter into a large vein in the arm, neck or groin and guiding it through the right side of the heart into the pulmonary artery. This helps measure the blood pressure in the lungs.
Specialized tests (if indicated)
Genetic testing: In pulmonary arterial hypertension (PAH), genetic testing is advised to detect pathogenic variants, most frequently in the BMPR2 gene, which can validate heritable forms of the condition and direct family screening and treatment. Given that 10-15% of idiopathic PAH cases have a heritable genetic basis, genetic counselling and gene panel testing need to be made available to all patients with idiopathic, familial, or congenital heart disease-associated PAH.
Lung biopsy: Although lung biopsies are rarely used to diagnose pulmonary hypertension, they may be taken into consideration to rule out other parenchymal lung diseases or when the cause of the condition is still unknown following noninvasive evaluation. Lung biopsies in PH patients are extremely risky and should only be performed in certain situations where histopathological confirmation is crucial, even though different biopsy techniques have high diagnostic accuracy for pulmonary lesions.
Stages of Pulmonary Hypertension
Based on data taken from the executive summary of the 1998 World Symposium on Primary Pulmonary Hypertension in Evian, France, the following are the 4 stages of pulmonary hypertension:
Class I: Patients have pulmonary hypertension, but are not subsequently limited in their physical activity. Patients who engage in regular physical exercise do not experience near-syncope, chest pain, fatigue or dyspnoea.
Class II: Patients have pulmonary hypertension, which causes them to be slightly less active. While at rest, the patients are comfortable, but during regular physical activity, they have near-syncope, chest pain, fatigue or dyspnoea.
Class III: Patients have pulmonary hypertension, which significantly restricts their ability to engage in physical exercise. Even less-than-normal activity can result in near-syncope, chest pain, fatigue or dyspnoea, even while patients are comfortable at rest.
Class IV: Patients who have pulmonary hypertension and are unable to exercise at all without experiencing symptoms. These individuals exhibit symptoms of right-sided heart failure, and any physical activity exacerbates their discomfort. Dyspnoea or exhaustion may even be present while they are at rest.
Pulmonary Hypertension Differential Diagnosis
A Differential diagnosis is a list of possible medical conditions or diseases that can share the same symptoms in a person. Pulmonary hypertension disease has an extensive differential diagnosis; the following are some of the conditions included in the differential diagnosis of pulmonary hypertension:
- Aortic stenosis: It is characterized by blood flow obstruction across the aortic valve. Chest pain is typically precipitated by exertion and relieved by rest.
- Chronic obstructive pulmonary disease (COPD): It is defined as damage to the lungs' airways or other components. It is characterized by an elevated jugular venous pulse (JVP).
- Restrictive lung disease: It occurs when the lungs struggle to expand completely during inhalation, resulting in a reduction in lung volume. Progressive exertional dyspnea is the predominant symptom.
- Mitral regurgitation: Partial closure of the mitral valve causes blood to backflow from the left ventricle into the left atrium during systole. Pulmonary edema is often the initial manifestation.
- Restrictive cardiomyopathy: It is a cardiac disorder in which the heart muscle becomes stiff, preventing the ventricles (lower chambers of the heart) from filling with blood properly. Patients may have a history of syncopal attacks due to a variety of causes, but orthostatic hypotension secondary to peripheral and/or autonomic neuropathy should be excluded.
- Hypertrophic cardiomyopathy: It is a hereditary cardiac disorder in which unusually thick heart muscles restrict the heart's ability to pump blood. Typical symptoms of angina are quite common in patients with HCM and occur in the absence of detectable coronary atherosclerosis.
- Dilated cardiomyopathy: It is a disorder in which the heart muscle enlarges and weakens, making it difficult for the heart to pump blood efficiently. Paroxysmal nocturnal dyspnea is a good indicator of the severity of DCM.
Goals of Treatment of Pulmonary Hypertension
The main goals of the treatment of pulmonary hypertension include:
- To enhance quality of life
- To stop progression of the disease
- To improve functional capacity
- To support heart function
To address pulmonary hypertension, rehabilitation programs and routine physical therapy are crucial. They help patients in increasing their level of activity, which can strengthen their hearts, increase their capacity for exercise, and enhance general health.
Although there is no cure for pulmonary hypertension, therapy can help manage the illness better and reduce symptoms. Usually getting worse over time, pulmonary hypertension can cause potentially fatal heart failure if ignored. As a result, initiating treatment as soon as possible is essential. Below are the treatment options for pulmonary hypertension:
- Nonpharmacological treatment of PH
- Pharmacological treatment of PH
- Surgical management of PH
- Other procedures
Nonpharmacological Treatment of PH
The goal of nonpharmacological therapy for pulmonary hypertension (PH) is to improve overall health and quality of life by modifying lifestyle choices and incorporating complementary therapies along with conventional medicine. These include dietary adjustments, trigger avoidance techniques, and physical activity in general; pulmonologists may recommend:
- Dietary changes: Limiting salt intake, avoiding saturated and trans fats, and focusing on whole grains, lean proteins, fruits, and vegetables.
- Lifestyle changes: Regular exercise, even low-intensity activities, can improve lung function and reduce symptoms. However, it's essential to consult with a pulmonologist or a specialist in pulmonary hypertension for a personalized and safe exercise plan.
- Oxygen therapy: For those people with pulmonary hypertension, oxygen therapy is a crucial supportive treatment, particularly for those who are hypoxic (have low blood oxygen levels). Its main objectives were to increase blood oxygen saturation, alleviate symptoms including exhaustion and shortness of breath, and reduce the likelihood of readmissions to the hospital. Oxygen therapy significantly improved the patient's quality of life and overall health, despite not directly treating pulmonary hypertension.
Pharmacological Treatment of PH
Improving blood flow to the lungs and lowering pulmonary artery pressure are the goals of pharmacological treatment for pulmonary hypertension. Medicines to treat pulmonary hypertension may include:
- Calcium channel blockers: These medications help lower blood pressure by relaxing the blood vessels; they are typically used in a small group of patients who show a favorable response to vasodilator testing during cardiac catheterization.
- Digitalis glycosides: These drugs help improve the heart's pumping ability by increasing the force of heart contractions, which is especially beneficial for patients with heart failure or poor heart function due to pulmonary hypertension.
- Diuretics: Helps eliminate fluid overload from the body, reducing strain on the heart and decreasing the fluid accumulation in the lungs, which can occur with pulmonary hypertension. This helps lower the pressure in the pulmonary arteries and improves overall cardiac function.
- Oxygen: Oxygen therapy, typically administered through a nasal cannula or face mask, is used to increase oxygen levels in the blood. It helps lower symptoms such as shortness of breath and decreases the strain on the heart in patients with low oxygen saturation because of pulmonary hypertension.
- Anticoagulant: an anticoagulant that prevents blood clotting. It is often prescribed to reduce the risk of blood clots, which can form more easily in individuals with pulmonary hypertension, especially those with underlying heart or lung disease.
- Endothelin receptor antagonists: which help to prevent blood vessels from narrowing.
- Phosphodiesterase inhibitors (PDE 5 inhibitors): These allow the lungs to produce more of their own natural vasodilators.
- Endothelin receptor antagonist combined with PDE5: Although they do so in different ways, the two drugs in this single-dose tablet lower blood pressure in the arteries of the lungs.
- Prostacyclin Analogue: This helps reduce the pressure in the lungs' blood vessels, making it easier for blood to flow through them and improving overall heart function.
- Selective IP receptor agonist: By activating the prostacyclin receptor, it promotes the dilation of pulmonary blood vessels, thereby improving blood flow and decreasing the heart's workload.
- Soluble guanylate cyclase stimulators: Increase the interactivity of the sGC enzyme with another chemical to help the blood vessels in the lungs relax
Women of childbearing age who have pulmonary arterial hypertension (PAH) should be advised not to become pregnant because of the high risks to both mother and baby. They should receive clear information about effective contraception and be offered psychosocial support.
If a woman with PAH wishes to become pregnant or becomes pregnant, she should receive counselling at a specialized pulmonary hypertension centre. This counselling should include discussions about the risks of pregnancy, available treatment options, and genetic counselling. Women can also be informed about alternatives such as adoption and surrogacy, and preconception genetic counselling can help them understand inherited risks.
Surgical Management of Pulmonary Hypertension
Some people with pulmonary hypertension who do not respond to medication may indeed require surgery. The types of surgery currently used for pulmonary hypertension include:
- Pulmonary endarterectomy: It is a surgical procedure performed to remove chronic blood clots and obstructive material from the pulmonary arteries in individuals with chronic thromboembolic pulmonary hypertension (CTEPH). The goal of the surgery is to reduce pulmonary artery pressure and improve respiratory function by restoring normal blood flow through the lungs.
- Balloon pulmonary angioplasty: This procedure uses a small balloon to widen blocked pulmonary arteries and restore blood flow. It is an alternative for patients unsuitable for pulmonary endarterectomy and helps reduce lung pressure, improve breathing, and increase exercise capacity.
- Balloon atrial septostomy: A procedure where a small hole is created between the left and right atria using a cardiac catheter. It helps to reduce pressure on the right side of the heart, improving blood flow to the lungs and heart efficiency.
- Transplant: Although a lung or heart-lung transplant may be necessary in extreme circumstances, this surgical alternative is rarely necessary because there are now efficient medicinal treatments available.
Pulmonary Hypertension Prognosis
The prognosis of pulmonary hypertension (PH) depends on the underlying cause, disease severity, and the response to treatment. If untreated, pulmonary arterial hypertension (PAH) can lead to right heart failure and death, with 1-year mortality rates of 8% for intermediate-risk and 19% for high-risk patients. Three-year mortality rates rise to 20% and 55%, respectively. Early diagnosis and treatment are important for effective management. Prognosis varies by PAH subtype, with right ventricular dysfunction in left heart disease (LHD) and PH in lung disease linked to higher mortality. Even mild PH in lung disease worsens survival and increases the risk of hospitalization. Chronic thromboembolic PH (CTEPH) has a good long-term outlook if operable.
Frequently Asked Questions (FAQs) on Pulmonary Hypertension
Is pulmonary hypertension curable?
While pulmonary hypertension is typically not curable, treatments can significantly alleviate symptoms and help extend life expectancy. By managing the condition effectively, patients can experience improved quality of life and better heart and lung function over time.
What medications can cause pulmonary hypertension?
Several medications have been linked to the development of pulmonary hypertension, particularly pulmonary arterial hypertension (PAH). These include certain appetite suppressants, recreational drugs, some cancer treatments, and specific antidepressants such as selective serotonin reuptake inhibitors (SSRIs).
Is pulmonary hypertension hereditary?
Yes, pulmonary hypertension can be inherited. This condition is known as heritable pulmonary arterial hypertension (HPAH) and is caused by mutations in certain genes. One of the most common genes associated with inherited pulmonary arterial hypertension (PAH) is BMPR2, with mutations in this gene found in approximately 70% of inherited cases.
How to avoid pulmonary hypertension?
Following a healthy lifestyle is crucial in managing and reducing the risk of pulmonary hypertension. This includes maintaining a balanced diet, exercising regularly, and keeping a healthy body mass index (BMI). Avoiding tobacco is especially important, as smoking significantly increases the risk of pulmonary hypertension and other cardiovascular conditions.
Who treats pulmonary hypertension?
Pulmonary hypertension is a complex medical condition that typically requires the expertise of both pulmonologists (lung specialists) and cardiologists (heart specialists). These specialists often collaborate to provide comprehensive care, and patients are frequently referred to specialized centres for advanced evaluation and treatment of pulmonary hypertension.
Can sleep apnea cause pulmonary hypertension?
Obstructive sleep apnea (OSA) can contribute to the development of pulmonary hypertension (PH) by repeatedly reducing oxygen levels during sleep. This chronic drop in oxygen causes the blood vessels in the lungs to constrict, which in turn raises the pressure within the pulmonary arteries. Over time, this sustained strain can lead to the progression of PH, a serious condition that can impair heart and lung function.
How to treat pulmonary hypertension?
Treatment for pulmonary hypertension targets the underlying cause, improves blood flow, and eases symptoms. It typically involves medications to reduce pressure, lifestyle modifications, and, in some cases, oxygen therapy or surgical options to support heart and lung function. The aim is to improve quality of life and slow the progression of the disease.
Can pulmonary hypertension cause death?
Untreated pulmonary hypertension can be life-threatening. The increased pressure in the lungs puts extra strain on the heart, which may lead to right-sided heart failure and, in severe cases, can be fatal.
Can ECG detect pulmonary hypertension?
An electrocardiogram (ECG) can suggest pulmonary hypertension (PH) but isn’t definitive due to limited accuracy. Further tests are usually needed for confirmation. If left untreated, PH can be life-threatening, potentially leading to right-sided heart failure.
Does pulmonary hypertension cause high blood pressure?
Pulmonary hypertension causes high blood pressure by affecting the arteries in the lungs and the right side of the heart. It happens when the blood pressure in the vessels that carry blood from the heart to the lungs becomes too high. This condition differs from regular high blood pressure, which affects the entire body.
Is mild pulmonary hypertension severe?
Mild pulmonary hypertension is not as severe as advanced stages, but it still needs medical attention. If left untreated, it can worsen over time and may impact on the patient's quality of life, as well as their lifespan in some cases. Identifying it early and receiving the proper care can help prevent problems and lead to improved health.
Can a CT scan detect pulmonary hypertension?
Yes, a CT scan, especially a CT pulmonary angiogram (CTPA), can be beneficial in diagnosing and evaluating pulmonary hypertension. It can help identify possible causes of the condition and assist pulmonologists in understanding its severity.
Can a heart transplant cure pulmonary hypertension?
Pulmonary arterial hypertension is a condition with limited treatment options that improve survival, and transplantation remains the only curative approach. Although data comparing lung and heart-lung transplants are limited, most evidence shows that patients with severe right ventricular failure often recover heart function after a double-lung transplant. In the absence of complex heart conditions, double-lung transplantation is generally the preferred surgical option.
How to measure pulmonary hypertension?
Pulmonary hypertension (PH) is assessed using right heart catheterization and echocardiography. Right heart catheterization is the gold standard, as it directly measures pressures in the heart and lungs. Echocardiography is a noninvasive method that estimates pulmonary artery pressures, particularly systolic pressure.